Catalog No.
YHK77701
Expression system
E. coli
Species
Homo sapiens (Human)
Protein length
Lys113-Ser275
Predicted molecular weight
40.47 kDa
Nature
Recombinant
Endotoxin level
Please contact with the lab for this information.
Purity
>90% as determined by SDS-PAGE.
Accession
Q9NQT5
Applications
ELISA, Immunogen, SDS-PAGE, WB, Bioactivity testing in progress
Form
Lyophilized
Storage buffer
Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Reconstitution
Reconstitute in sterile water for a stock solution. A copy of datasheet will be provided with the products, please refer to it for details.
Shipping
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
Alternative Names
EXOSC3, Exosome component 3, Exosome complex component RRP40, RRP40, Ribosomal RNA-processing protein 40, p10
EXOSC3 S1-domain variants implicated in PCH1b alter RNA exosome cap subunit abundance and thermal stability disrupting rRNA processing and targeting of AU-rich mRNA., PMID:40501579
The First Known Case Report of a Novel Homozygous Nonsense Variant in the OSBPL9 Gene Associated With Fetal Cerebral Ventriculomegaly, Cerebellar Hypoplasia, and Arthrogryposis Multiplex., PMID:40182349
Potentially functional variants of INPP5D and EXOSC3 in immunity B cell-related genes are associated with non-small cell lung cancer survival., PMID:39176091
Mutations in Genes Encoding Subunits of the RNA Exosome as a Potential Novel Cause of Thrombotic Microangiopathy., PMID:39062862
Elucidating the EXOSC3-IRE1α interaction: a convergent study incorporating computational, in vitro and in vivo studies., PMID:38407190
The putative RNA helicase DDX1 associates with the nuclear RNA exosome and modulates RNA/DNA hybrids (R-loops)., PMID:38219817
Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study., PMID:37369098
Generation of an iPSC line from a Pontocerebellar Hypoplasia 1B patient harboring a homozygous c.395 A > C mutation in EXOSC3 along with a family matched control., PMID:36257093
Identification of Hub Genes of Lung Adenocarcinoma Based on Weighted Gene Co-Expression Network in Chinese Population., PMID:36032660
Molecularly confirmed pontocerebellar hypoplasia in a large family from Slovakia with four severely affected children., PMID:35852507
RNA exosome drives early B cell development via noncoding RNA processing mechanisms., PMID:35658015
Atypical hemolytic uremic syndrome induced by SARS-CoV2 infection in infants with EXOSC3 mutation., PMID:35522339
Construction and Validation of a Newly Prognostic Signature for CRISPR-Cas9-Based Cancer Dependency Map Genes in Breast Cancer., PMID:35096059
Detection of Melanogenesis and Anti-Apoptosis-Associated Melanoma Factors: Array CGH and PPI Mapping Integrating Study., PMID:34749602
Aberrant expression of MYD88 via RNA-controlling CNOT4 and EXOSC3 in colonic mucosa impacts generation of colonic cancer., PMID:34626022
Risk of sudden cardiac death in EXOSC5-related disease., PMID:34089229
Refining the mutational spectrum and gene-phenotype correlates in pontocerebellar hypoplasia: results of a multicentric study., PMID:34085948
Bi-allelic missense variant, p.Ser35Leu in EXOSC1 is associated with pontocerebellar hypoplasia., PMID:33463720
Two siblings with a novel variant of EXOSC3 extended phenotypic spectrum of pontocerebellar hypoplasia 1B to an exceptionally mild form., PMID:33462000
A Drosophila model of Pontocerebellar Hypoplasia reveals a critical role for the RNA exosome in neurons., PMID:32645003
RNA exosome mutations in pontocerebellar hypoplasia alter ribosome biogenesis and p53 levels., PMID:32527837
Osteopetrosis-Associated Transmembrane Protein 1 Recruits RNA Exosome To Restrict Hepatitis B Virus Replication., PMID:32188736
Pontocerebellar hypoplasia with rhombencephalosynapsis and microlissencephaly expands the spectrum of PCH type 1B., PMID:31770597
Infantile onset progressive cerebellar atrophy and anterior horn cell Degeneration-A novel phenotype associated with mutations in the PLA2G6 gene., PMID:31689548
Genome Resequencing Reveals Congenital Causes of Embryo and Nestling Death in Crested Ibis (Nipponia nippon)., PMID:31298688
Novel EXOSC3 pathogenic variant results in a mild course of neurologic disease with cerebellum involvement., PMID:30986545
Posttranscriptional modulation of TERC by PAPD5 inhibition rescues hematopoietic development in dyskeratosis congenita., PMID:30728146
A Chemical Biology Approach to Model Pontocerebellar Hypoplasia Type 1B (PCH1B)., PMID:30141626
mRNAs are sorted for export or degradation before passing through nuclear speckles., PMID:30032211
Sarcomeric disorganization and nemaline bodies in muscle biopsies of patients with EXOSC3-related type 1 pontocerebellar hypoplasia., PMID:30025162
Variants in EXOSC9 Disrupt the RNA Exosome and Result in Cerebellar Atrophy with Spinal Motor Neuronopathy., PMID:29727687
Pontocerebellar hypoplasia type 1 for the neuropediatrician: Genotype-phenotype correlations and diagnostic guidelines based on new cases and overview of the literature., PMID:29656927
The RNA exosome and RNA exosome-linked disease., PMID:29093021
ARS2 is a general suppressor of pervasive transcription., PMID:28973446
Recessive mutation in EXOSC3 associates with mitochondrial dysfunction and pontocerebellar hypoplasia., PMID:28687512
The RNA Exosome Syncs IAV-RNAPII Transcription to Promote Viral Ribogenesis and Infectivity., PMID:28475896
Mutations of EXOSC3/Rrp40p associated with neurological diseases impact ribosomal RNA processing functions of the exosome in S. cerevisiae., PMID:28053271
[Familial EXOSC3-related pontocerebellar hypoplasia]., PMID:27876572
Insight into the RNA Exosome Complex Through Modeling Pontocerebellar Hypoplasia Type 1b Disease Mutations in Yeast., PMID:27777260
Altered RNA metabolism due to a homozygous RBM7 mutation in a patient with spinal motor neuropathy., PMID:27193168
Mutations in EXOSC2 are associated with a novel syndrome characterised by retinitis pigmentosa, progressive hearing loss, premature ageing, short stature, mild intellectual disability and distinctive gestalt., PMID:26843489
Nuclear matrix protein Matrin 3 is a regulator of ZAP-mediated retroviral restriction., PMID:26129669
RNA exosome-regulated long non-coding RNA transcription controls super-enhancer activity., PMID:25957685
Human Mendelian diseases related to abnormalities of the RNA exosome or its cofactors., PMID:25343120
Protein deep sequencing applied to biobank samples from patients with pancreatic cancer., PMID:25216700
Novel EXOSC3 mutation causes complicated hereditary spastic paraplegia., PMID:25149867
Noncoding RNA transcription targets AID to divergently transcribed loci in B cells., PMID:25119026
EXOSC3 mutations in pontocerebellar hypoplasia type 1: novel mutations and genotype-phenotype correlations., PMID:24524299
Exome sequencing in a family with intellectual disability, early onset spasticity, and cerebellar atrophy detects a novel mutation in EXOSC3., PMID:23975261
Homozygous EXOSC3 mutation c.92G→C, p.G31A is a founder mutation causing severe pontocerebellar hypoplasia type 1 among the Czech Roma., PMID:23883322
For research use only. Not for human or drug use.
